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familiaire ziekte van Creutzfeldt-Jakob (aandoening)
familiaire ziekte van Creutzfeldt-Jakob
fCJD
Familial Creutzfeldt-Jakob
Inherited Creutzfeldt-Jakob disease
fCJD (Familial Creutzfeldt-Jakob disease)
Inherited or familial Creutzfeldt-Jakob disease (fCJD) is a very rare form of genetic prion disease characterized by typical CJD features (rapidly progressive dementia, personality/behavioral changes, psychiatric disorders, myoclonus, and ataxia) with a genetic cause and sometimes a family history of dementia.
Id715807002
StatusPrimitive
Associated morphologyspongiforme degeneratie
Causative agentCreutzfeldt-Jakob agens
Finding sitestructuur van encephalon
Pathological processinfectieus proces
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetA81.0
TermZiekte van Creutzfeldt-Jakob
SNOMED CT to Orphanet simple map282166
SNOMED CT to ICD-10 extended map
TargetA81.0
RuleTRUE
AdviceALWAYS A81.0
CorrelationSNOMED CT source code to target map code correlation not specified